Islamagič Lejla, Ulhøi Benedicte Parm, von Oettingen Gorm, Cortnum Søren Ole Stigaard, Korshøj Anders Rosendal, Schechtmann Gaston
Department of Neurosurgery, Odense University Hospital, Odense, Denmark.
Department of Neurosurgery, Aarhus University Hospital, Aarhus, Denmark.
Acta Neurochir (Wien). 2025 Jul 10;167(1):187. doi: 10.1007/s00701-025-06606-4.
Rhombencephalitis (RE) or brainstem encephalitis is a rare but potentially deadly condition. It often affects hosts with weaker or altered immune response. A previously healthy 30-year-old female, three months post-partum, presented with a ten-day history of headache, vomiting, seizures and consciousness deterioration. MRI showed suspect brainstem glioma and hydrocephalus. Initial treatment aimed to reduce high intracranial pressure with an external ventricular drain and high-dose steroids. Despite negative blood cultures, CSF sampling was overlooked as RE was not suspected. The condition proved fatal four days later. Post-mortem histology revealed the RE as the cause of death, but PCR did not show an infectious ethology. With patients in the post-partum period with progressive neurological deterioration and FLAIR2-changes in brainstem MRI, RE should be considered early on.
菱脑炎（RE）或脑干脑炎是一种罕见但可能致命的疾病。它通常影响免疫反应较弱或改变的宿主。一名此前健康的30岁产后女性，产后三个月出现头痛、呕吐、癫痫发作和意识恶化的十天病史。磁共振成像（MRI）显示疑似脑干胶质瘤和脑积水。初始治疗旨在通过外部脑室引流和大剂量类固醇降低高颅内压。尽管血培养结果为阴性，但由于未怀疑RE，脑脊液采样被忽视。四天后该病情被证明是致命的。尸检组织学显示RE是死亡原因，但聚合酶链反应（PCR）未显示感染病因。对于产后出现进行性神经功能恶化且脑干MRI有液体衰减反转恢复序列（FLAIR）改变的患者，应尽早考虑RE。
